An Unusual Cause of an Uncommon Condition: Pulmonary Vein Stenosis

Pulmonary vein stenosis in adults is historically a rare condition, but is becoming a recognised complication, albeit an uncommon one, of radiofrequency ablation around the pulmonary veins for treatment of atrial fibrillation. It may also be due to infiltrating mediastinal processes such as neoplasm or sarcoidosis. In this case report, a 55-year-old man underwent resection of a mediastinal phaeochromocytoma involving the left atrial wall and the right inferior pulmonary vein. One year later he subsequently presented with increasing dyspnoea and atypical chest pain. Transthoracic echocardiogram showed severe pulmonary hypertension, right ventricular dilatation and dysfunction. Transesophageal echocardiogram demonstrated severe bilateral pulmonary vein stenosis with peak/mean gradients across the left pulmonary veins of about 25/20mmHg. The diagnosis was also confirmed on CT pulmonary angiography with 3D reconstruction. Open pulmonary vein stenting was planned but unfortunately the patient died suddenly before the procedure. Pulmonary vein stenosis is an uncommon but serious condition and may present with signs and symptoms indistinguishable from other conditions and may easily be missed. Clinicians should have a high index of suspicion when patients present with unexplained respiratory symptoms, especially in the context of catheter ablation or mediastinal processes such as neoplasm. Transesophageal echocardiography played an indispensible part in the correct diagnosis in our patient.